Pediatrics Commons^™

Who Speaks For Me?: Addressing Variability In Informed Consent Practices For Minimal Risk Research Involving Foster Youth, Mary V. Greiner, Sarah J. Beal, Antonio Allen, Vikash Patel, Jareen Meinzen-Derr, Armand Antommaria

Journal of Health Disparities Research and Practice

Background: Youth in protective custody (i.e., foster care) are at higher risk for poorer physical and mental health outcomes compared with those who are not. These differences may be due in part to the lack of research on the population to create evidence-based recommendations for health care delivery. A potential contributor to this lack of research is difficulties in obtaining informed consent for empirical studies in this population. The objective of this study was to describe the approaches to obtaining informed consent in minimal risk studies of foster youth and provide recommendations for future requirements.

Methods: We conducted a …

Integrating Nutrition Into Health Systems: What The Evidence Advocates, Rehana Salam, Jai K. Das, Zulfiqar Ahmed Bhutta

Department of Paediatrics and Child Health

There is considerable evidence of positive health and nutrition outcomes resulting from integrating nutrition-specific interventions into health systems; however, current knowledge on establishing and sustaining effective integration of nutrition into health systems is limited. The objective of this review is to map the existing types of integration platforms and review the evidence on integrated health and nutrition programmes' impacts on specific nutrition outcomes. A literature search was conducted, and integrated nutrition programmes were examined through the lens of the six World Health Organization (WHO) building blocks, including the demand side. Forty-five studies were included in this review, outlining the integration …

Mutational Analysis Of Head And Neck Squamous Cell Carcinoma Stratified By Smoking Status., Farhad Ghasemi, Stephenie D Prokopec, Danielle Macneil, Neil Mundi, Steven F Gameiro, Christopher Howlett, William Stecho, Paul Plantinga, Nicole Pinto, Kara M Ruicci, Mohammed Imran Khan, John Yoo, Kevin Fung, Axel Sahovaler, David A Palma, Eric Winquist, Joe S Mymryk, John W Barrett, Paul C Boutros, Anthony C Nichols

Paediatrics Publications

Smoking has historically been recognized as a negative prognostic factor in head and neck squamous cell carcinoma (HNSCC). This study aimed to assess the mutational differences between heavy smokers (>20 pack years) and never smokers among the HNSCC patients within The Cancer Genome Atlas (TCGA). Single nucleotide variation and copy number aberration differences between heavy smokers and never smokers were compared within human papillomavirus-positive (HPV-positive) (n = 67) and HPV-negative (n = 431) TCGA cohorts with HNSCC, and the impact of these mutations on survival were assessed. No genes were differentially mutated between smoking and never-smoking patients with HPV-positive …

Csf Inflammatory Markers Differ In Gram-Positive Versus Gram-Negative Shunt Infections., Gwenn L. Skar, David C. Synhorst, Matthew Beaver, Jessica N. Snowden

Manuscripts, Articles, Book Chapters and Other Papers

Background: Cerebrospinal fluid (CSF) shunt placement is frequently complicated by bacterial infection. Shunt infection diagnosis relies on bacterial culture of CSF which can often produce false-negative results. Negative cultures present a conundrum for physicians as they are left to rely on other CSF indices, which can be unremarkable. New methods are needed to swiftly and accurately diagnose shunt infections. CSF chemokines and cytokines may prove useful as diagnostic biomarkers. The objective of this study was to evaluate the potential of systemic and CSF biomarkers for identification of CSF shunt infection.

Methods: We conducted a retrospective chart review of children with …

Oral Polio Vaccine Response In The Mal-Ed Birth Cohort Study: Considerations For Polio Eradication Strategies, William K. Pan, Jessica C. Seidman, Syed Asad Ali, Christel Hoest, Carl Mason, Dinesh Mondal, Stacey L. Knobler, Pascal Bessong, Mal-Ed Network Investigators

Department of Paediatrics and Child Health

Background: Immunization programs have leveraged decades of research to maximize oral polio vaccine (OPV) response. Moving toward global poliovirus eradication, the WHO recommended phased OPV-to-IPV replacement on schedules in 2012. Using the MAL-ED prospective birth cohort data, we evaluated the influence of early life exposures impacting OPV immunization by measuring OPV response for serotypes 1 and 3.
Methods: Polio neutralizing antibody assays were conducted at 7 and 15 months of age for serotypes 1 and 3. Analyses were conducted on children receiving ≥3 OPV doses (n = 1449). History of vaccination, feeding patterns, physical growth, home environment, diarrhea, enteropathogen detection, …

Exploring In Silence: Hearing And Deaf Infants Explore Objects Differently Before Cochlear Implantation, Mary K. Fagan

Communication Sciences and Disorders Faculty Articles and Research

Infant development has rarely been informed by the behavior of infants with sensory differences despite increasing recognition that infant behavior itself creates sensory learning opportunities. The purpose of this study of object exploration was to compare the behavior of hearing and deaf infants, with and without cochlear implants, in order to identify the effects of profound sensorineural hearing loss on infant exploration before cochlear implantation, the behavioral effects of access to auditory feedback after cochlear implantation, and the sensory motivation for exploration behaviors performed by hearing infants as well. The results showed that 9-month-old deaf infants explored objects as often …

Cdk4 Inhibitors Thwart Immunity By Inhibiting Phospho-Rb-Nf-Kb Complexes, Seung J. Kim, Samuel Asfaha, Frederick A. Dick

Paediatrics Publications

PD-L1 plays a central role in immune recognition of cancer cells. In this issue of Molecular Cell, Jin et al. (2019) report that a phosphorylated retinoblastoma protein contacts the DNA-binding domain of p65 NF-kB, thereby blocking transcription of PD-L1.

Ctcf Governs The Identity And Migration Of Mge-Derived Cortical Interneurons., Adrienne Elbert, Daniel Vogt, Ashley Watson, Michael Levy, Yan Jiang, Emilie Brûlé, Megan E Rowland, John Rubenstein, Nathalie G Bérubé

Paediatrics Publications

The CCCTC-binding factor (CTCF) is a central regulator of chromatin topology recently linked to neurodevelopmental disorders such as intellectual disability, autism, and schizophrenia. The aim of this study was to identify novel roles of CTCF in the developing mouse brain. We provide evidence that CTCF is required for the expression of the LIM homeodomain factor LHX6 involved in fate determination of cortical interneurons (CINs) that originate in the medial ganglionic eminence (MGE). Conditional

A State-Level Study Of Opioid Use Disorder Treatment Access And Neonatal Abstinence Syndrome, Elizabeth R. Wolf, Sebastian T. Tong, Roy T. Sabo, Steven H. Woolf, Kassie Abbinanti, James Pecsok, Alex H. Krist

Pediatrics Publications

Background Adult opioid use and neonatal abstinence syndrome (NAS) are growing public health problems in the United States (U.S.). Our objective was to determine how opioid use disorder treatment access impacts the relationship between adult opioid use and NAS.

Methods We conducted a cross-sectional state-level ecologic study using 36 states with available Healthcare Cost and Utilization Project State Inpatient Databases in 2014. Opioid use disorder treatment access was determined by the: 1) proportion of people needing but not receiving substance use treatment, 2) density of buprenorphine-waivered physicians, and 3) proportion of individuals in outpatient treatment programs (OTPs). The incidence of …

Alternative Care Models For Management Of Diabetes, Sarah Tsai

Presentations

Describes effort to improve A1C in teens using motivational interviewing vs. traditional patient/provider interaction in real-world clinic setting.

Prevalence Of Cardiovascular Disease Risk Factors In Childhood Glomerular Diseases, I. F. Ashoor, S. A. Mansfield, M. M. O'Shaughnessy, R. S. Parekh, J. Zee, T. L. Vasylyeva, A. J. Kogon, C. B. Sethna, L. M. Guay-Woodford, +170 Additional Authors

Journal Articles

No abstract provided.

Hydroxyurea For Children With Sickle Cell Anemia In Sub-Saharan Africa, L. Tshilolo, G. Tomlinson, T. N. Williams, B. Santos, P. Olupot-Olupot, A. Lane, B. Aygun, S. E. Stuber, K. Mcelhinney, +59 Additional Authors

Journal Articles

No abstract provided.

Psychosocial Screening In Disorders/Differences Of Sex Development: Psychometric Evaluation Of The Psychosocial Assessment Tool, M. M. Ernst, M. Gardner, C. A. Mara, E. C. Délot, P. Y. Fechner, M. Fox, M. M. Rutter, P. W. Speiser, C. Foley, B. Yashar, +24 Additional Authors

Journal Articles

© 2019 S. Karger AG, Basel. Background/Aims: Utilization of a psychosocial screener to identify families affected by a disorder/difference of sex development (DSD) and at risk for adjustment challenges may facilitate efficient use of team resources to optimize care. The Psychosocial Assessment Tool (PAT) has been used in other pediatric conditions. The current study explored the reliability and validity of the PAT (modified for use within the DSD population; PAT-DSD). Methods: Participants were 197 families enrolled in the DSD-Translational Research Network (DSD-TRN) who completed a PAT-DSD during a DSD clinic visit. Psychosocial data were extracted from the DSD-TRN clinical registry. …

A Prospective Investigation Of Interleukin-8 Levels In Pediatric Acute Respiratory Failure And Acute Respiratory Distress Syndrome, H. Flori, A. Sapru, M. W. Quasney, G. Gildengorin, M. A. Curley, M. A. Matthay, M. K. Dahmer, S. T. Bateman, J. B. Schneider, D. Wypij, +20 Additional Authors

Journal Articles

© 2019 The Author(s). Background: The association of plasma interleukin-8 (IL-8), or IL-8 genetic variants, with pediatric acute respiratory distress syndrome (PARDS) in children with acute respiratory failure at risk for PARDS has not been examined. The purpose of this study was to examine the association of early and sequential measurement of plasma IL-8 and/or its genetic variants with development of PARDS and other clinical outcomes in mechanically ventilated children with acute respiratory failure. Methods: This was a prospective cohort study of children 2 weeks to 17 years of age with acute airways and/or parenchymal lung disease done in 22 …

Immunosuppressive Therapy For Pediatric Aplastic Anemia: A North American Pediatric Aplastic Anemia Consortium Study., Z. R. Rogers, T. A. Nakano, T. S. Olson, A. A. Bertuch, W. Wang, A. Gillio, T. D. Coates, A. Chawla, A. Vlachos, A. Shimamura, +19 Additional Authors

Journal Articles

Quality of response to immunosuppressive therapy and long-term outcomes for pediatric severe aplastic anemia remain incompletely characterized. Contemporary evidence to inform treatment of relapsed or refractory severe aplastic anemia for pediatric patients is also limited. The clinical features and outcomes for 314 children treated from 2002 to 2014 with immunosuppressive therapy for acquired severe aplastic anemia were analyzed retrospectively from 25 institutions in the North American Pediatric Aplastic Anemia Consortium. The majority of subjects (n=264) received horse anti-thymocyte globulin (hATG) plus cyclosporine (CyA) with a median 61 months follow up. Following hATG/CyA, 71.2% (95%CI: 65.3,76.6) achieved an objective response. In …

Plasma Zonulin Levels In Childhood Nephrotic Syndrome, H. Trachtman, D. S. Gipson, K. V. Lemley, J. P. Troost, C. Faul, D. J. Morrison, S. M. Vento, D. H. Ahn, C. Sethna, C. Avila-Casado, +90 Additional Authors

Journal Articles

© 2019 Trachtman, Gipson, Lemley, Troost, Faul, Morrison, Vento, Ahn and Goldberg. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. Objective: We conducted this study to test the hypothesis that plasma zonulin levels are elevated in pediatric patients with nephrotic syndrome …

Text Messaging For Disease Monitoring In Childhood Nephrotic Syndrome, C. S. Wang, J. P. Troost, L. A. Greenbaum, T. Srivastava, K. Reidy, K. Gibson, H. Trachtman, J. D. Piette, C. B. Sethna, D. S. Gipson, +12 Additional Authors

Journal Articles

© 2019 International Society of Nephrology Introduction: There is limited information on effective disease monitoring for prompt interventions in childhood nephrotic syndrome. We examined the feasibility and effectiveness of a novel text messaging system (SMS) for disease monitoring in a multicenter, prospective study. Methods: A total of 127 patients results, symptoms, and medication adherence were sent to a designated caregiver (n = 116) or adolescent patient (n = 3). Participants responded by texting. Feasibility of SMS was assessed by SMS adoption, retention, and engagement, and concordance between participant-reported results and laboratory/clinician assessments. The number of disease relapses and time-to-remission data …

Serum S100a8/A9 And S100a12 Levels In Children With Polyarticular Forms Of Juvenile Idiopathic Arthritis: Relationship To Maintenance Of Clinically Inactive Disease During Anti–Tumor Necrosis Factor Therapy And Occurrence Of Disease Flare After Discontinuation Of Therapy, C. H. Hinze, D. Foell, A. L. Johnson, S. J. Spalding, B. S. Gottlieb, P. W. Morris, Y. Kimura, K. Onel, S. C. Li, D. J. Lovell, +33 Additional Authors

Journal Articles

© 2018, American College of Rheumatology Objective: To determine the relationship between serum levels of S100A8/A9 and S100A12 and the maintenance of clinically inactive disease during anti–tumor necrosis factor (anti-TNF) therapy and the occurrence of disease flare following withdrawal of anti-TNF therapy in patients with polyarticular forms of juvenile idiopathic arthritis (JIA). Methods: In this prospective, multicenter study, 137 patients with polyarticular-course JIA whose disease was clinically inactive while receiving anti-TNF therapy were enrolled. Patients were observed for an initial 6-month phase during which anti-TNF treatment was continued. For those patients who maintained clinically inactive disease over the 6 months, …

Correlates Of Genetic Attributions Among Parents Of Children In The Usa With Developmental Disabilities, B. Kiely, S. Vettam, A. Adesman

Journal Articles

© 2019 Kiely et al. php and incorporate. Introduction: As technologies for identifying causal genetic variants in children with autism spectrum disorders (ASD) and other developmental conditions continue to advance, there is a need to understand the factors that influence parental beliefs about the causes of their child’s disabilities. This study assessed the correlates of etiologic attributions among US parents of children with ASD, intellectual disability (ID), and/or developmental delay (DD). Methods: Data were obtained from the Centers for Disease Control and Prevention’s nationally representative Survey of Pathways to Diagnosis and Services. Respondents were classified according to whether their child …

Demographics And Anthropometrics Impact Benefits Of Health Intervention: Data From The Reduce Obesity And Diabetes Project., L. Ostrowski, P. W. Speiser, S. Accacha, L. Altshuler, I. Fennoy, B. Lowell, R. Rapaport, W. Rosenfeld, S. P. Shelov, Road Group, +2 Additional Authors

Journal Articles

Objective:To determine the efficacy of a 4-month school-based health, nutrition and exercise intervention on body fatness and examine possible effects of demographic and anthropometric covariates. Methods:Height, weight, waist circumference and body composition were measured in a diverse population of 644 NYC middle school students (mean ± SD age 12.7 ± 0.9 years; 46% male; 38% Hispanic, 17% East Asian, 15% South Asian, 13.5% African American, 8.5% Caucasian, 8% other) during the fall and spring semesters. Year 1 participants (n = 322) were controls. Experimental participants (year 2, n = 469) received a 12-session classroom-based health and nutrition educational programme with …

Emerging Medical Therapies For Congenital Adrenal Hyperplasia, P. W. Speiser

Journal Articles

© 2019 Speiser PW. Congenital adrenal hyperplasia has traditionally been treated with daily oral doses of glucocorticoids and mineralocorticoid supplements. Such therapy does not precisely replicate the adrenal cortex's circadian pattern. As a consequence, patients are intermittently overtreated or undertreated leading to growth suppression in children, excess weight gain and altered metabolism. Several new treatments are on the horizon. This article will summarize some new potential therapies as adjuncts to, or replacement for, standard therapy.

Sr-B1 Drives Endothelial Cell Ldl Transcytosis Via Dock4 To Promote Atherosclerosis, L. Huang, K. L. Chambliss, X. Gao, I. S. Yuhanna, E. Behling-Kelly, S. Bergaya, M. Ahmed, P. Michaely, K. Luby-Phelps, P. W. Shaul, +5 Additional Authors

Journal Articles

© 2019, The Author(s), under exclusive licence to Springer Nature Limited. Atherosclerosis, which underlies life-threatening cardiovascular disorders such as myocardial infarction and stroke1, is initiated by passage of low-density lipoprotein (LDL) cholesterol into the artery wall and its engulfment by macrophages, which leads to foam cell formation and lesion development2,3. It is unclear how circulating LDL enters the artery wall to instigate atherosclerosis. Here we show in mice that scavenger receptor class B type 1 (SR-B1) in endothelial cells mediates the delivery of LDL into arteries and its accumulation by artery wall macrophages, thereby promoting atherosclerosis. LDL particles are colocalized …

Caffeine Inhibits Hypoxia-Induced Nuclear Accumulation In Hif-1Α And Promotes Neonatal Neuronal Survival, H. L. Li, N. Zaghloul, I. Ahmed, A. Omelchenko, B. L. Firestein, H. Huang, L. Collins

Journal Articles

© 2019 The Authors Apnea of prematurity (AOP) defined as cessation of breathing for 15–20 s, is commonly seen in preterm infants. Caffeine is widely used to treat AOP due to its safety and effectiveness. Caffeine releases respiratory arrest by competing with adenosine for binding to adenosine A 1 and A 2A receptors (A 1 R and A 2A R). Long before its use in treating AOP, caffeine has been used as a psychostimulant in adult brains. However, the effect of caffeine on developing brains remains unclear. We found that A 1 R proteins for caffeine binding were expressed in …

Closed-Loop Cardiovascular Interactions And The Baroreflex Cardiac Arm: Modulations Over The 24 H And The Effect Of Hypertension, Gianfranco Parati, Paolo Castiglioni, Andrea Faini, Marco Di Rienzo, Giuseppe Mancia, Riccardo Barbieri, J. Philip Saul

Faculty & Staff Scholarship

Closed-loop models of the interactions between blood pressure (BP) and heart rate variations allow for estimation of baroreflex sensitivity (feedback effects of BP changes on heart rate) while also considering the feedforward effects of heart rate on BP. Our study is aimed at comparing modulations of feedback and feedforward couplings over 24 h in normotensive and hypertensive subjects, by assessing closed-loop baroreflex models in ambulatory conditions. Continuous intra-arterial BP recordings were performed for 24 h in eight normotensive and eight hypertensive subjects. Systolic BP (SBP) and pulse interval (PI) beat-by-beat series were analyzed by an autoregressive moving average model over …

Ampa Receptor Glua2 Subunit Defects Are A Cause Of Neurodevelopmental Disorders, V. Salpietro, C. L. Dixon, H. Guo, O. D. Bello, J. Vandrovcova, S. Efthymiou, R. Maroofian, S. Chen, L. Pisani, F. Madia, +90 Additional Authors

Journal Articles

© 2019, The Author(s). AMPA receptors (AMPARs) are tetrameric ligand-gated channels made up of combinations of GluA1-4 subunits encoded by GRIA1-4 genes. GluA2 has an especially important role because, following post-transcriptional editing at the Q607 site, it renders heteromultimeric AMPARs Ca2+-impermeable, with a linear relationship between current and trans-membrane voltage. Here, we report heterozygous de novo GRIA2 mutations in 28 unrelated patients with intellectual disability (ID) and neurodevelopmental abnormalities including autism spectrum disorder (ASD), Rett syndrome-like features, and seizures or developmental epileptic encephalopathy (DEE). In functional expression studies, mutations lead to a decrease in agonist-evoked current mediated by mutant subunits …

Author Correction: Chd3 Helicase Domain Mutations Cause A Neurodevelopmental Syndrome With Macrocephaly And Impaired Speech And Language., L. S. Blok, J. Rousseau, J. Twist, S. Ehresmann, M. Takaku, H. Venselaar, L. H. Rodan, M. G. Bialer, C. Moore, M. Blyth, +90 Additional Authors

Journal Articles

© 2019, The Author(s). The original version of this Article contained an error in the spelling of the author Laurence Faivre, which was incorrectly given as Laurence Faive. This has now been corrected in both the PDF and HTML versions of the Article.

De Novo Interstitial Deletion Of 9q In A Pediatric Patient With Global Developmental Delay., D. Keselman, R. Singh, N. Cohen, Z. Fefer

Journal Articles

Cytogenomic microarray (CMA) methodologies, including array comparative genomic hybridization (aCGH) and single-nucleotide polymorphism-detecting arrays (SNP-array), are recommended as the first-tier test for the evaluation of imbalances associated with intellectual disability, autism, and multiple congenital anomalies. The authors report on a child with global developmental delay (GDD) and a de novo interstitial 7.0 Mb deletion of 9q21.33q22.31 detected by aCGH. The patient that the authors report here is noteworthy in that she presented with GDD and her interstitial deletion is not inclusive of the 9q22.32 locus that includes the PTCH1 gene, which is implicated in Gorlin syndrome, or basal cell nevus …

Persistent Cloaca And Caudal Duplication In A Monovular Twin, A Rare Case Report, N. Cohen, M. N. Ahmed, R. Goldfischer, N. Zaghloul

Journal Articles

© 2019 Introduction: A cloaca occurs when genitourinary tract and bowel converge into a common channel. We report a case of partial caudal duplication, persistent cloaca and vestigial appendage in a monovular female twin infant. Presentation of case: This is a monochorinonic-diamniotic twin born at 36 weeks with apgars of 9/9. She had a duplicated labia with two clitorises, and a partially formed accessory foot with 2 toes protruding from the right gluteal region. There was anal atresia and a punctate urethral opening in the right genitalia through which she voided spontaneously. X-ray of the accessory foot had rudimentary metatarsals …

Quantitative Human Immunodeficiency Virus (Hiv)-1 Antibodies Correlate With Plasma Hiv-1 Rna And Cell-Associated Dna Levels In Children On Antiretroviral Therapy, M. Mcmanus, J. Henderson, A. Gautam, R. Brody, E. R. Weiss, D. Persaud, E. Mick, K. Luzuriaga, V. Bonagura, I. Febo, +22 Additional Authors

Journal Articles

No abstract provided.

Ulcerative Colitis Mucosal Transcriptomes Reveal Mitochondriopathy And Personalized Mechanisms Underlying Disease Severity And Treatment Response, Y. Haberman, R. Karns, P. J. Dexheimer, M. Schirmer, J. Somekh, I. Jurickova, T. Braun, E. Novak, J. Markowitz, L. A. Denson, +32 Additional Authors

Journal Articles

© 2019, The Author(s). Molecular mechanisms driving disease course and response to therapy in ulcerative colitis (UC) are not well understood. Here, we use RNAseq to define pre-treatment rectal gene expression, and fecal microbiota profiles, in 206 pediatric UC patients receiving standardised therapy. We validate our key findings in adult and paediatric UC cohorts of 408 participants. We observe a marked suppression of mitochondrial genes and function across cohorts in active UC, and that increasing disease severity is notable for enrichment of adenoma/adenocarcinoma and innate immune genes. A subset of severity genes improves prediction of corticosteroid-induced remission in the discovery …